Pure autonomic failure, previously called idiopathic orthostatic hypotension or Bradbury-Eggleston syndrome, denotes generalized autonomic failure without CNS involvement. This disorder differs from multiple system atrophy because it lacks central or preganglionic involvement. Pure autonomic failure affects more women, tends to begin during a person’s 40s or 50s, and does not result in death.

Etiology is usually unknown. Some cases are due to a synucleinopathy (see Cervical Dystonia : Pathophysiology); synuclein can also accumulate in patients with Parkinson disease, multiple system atrophy, or Lewy body dementia. Some patients with pure autonomic failure eventually develop multiple system atrophy or Lewy body dementia. Occasionally, the cause is an autoimmune autonomic neuropathy.

Symptoms

The main symptom is orthostatic hypotension; there may be other autonomic symptoms, such as decreased sweating, heat intolerance, urinary retention, bladder spasms (possibly causing incontinence), erectile dysfunction, fecal incontinence or constipation, and pupillary abnormalities.

Diagnosis

Clinical evaluation

Diagnosis is by exclusion. The norepinephrine level is usually < 100 pg/mL supine and does not increase with standing. Postural tachycardia syndrome can be differentiated because with standing, it does not usually cause hypotension, the norepinephrine level increases, and heart rate increases by > 30 beats/min or to 120 beats/min within 10 min.

MONDAY, July 20, 2015 (HealthDay News) -- Early data suggest a test based on a patient's saliva might someday help detect Alzheimer's disease. The study was to be presented Sunday at the annual Alzheimer's Association International Conference, held from July 18 to 23 in Washington, D.C.
Shraddha...

MONDAY, July 27, 2015 (HealthDay News) -- Praluent (alirocumab) injection has been approved by the U.S. Food and Drug Administration for use in addition to diet and maximally tolerated statin therapy in adults with heterozygous familial hypercholesterolemia or clinical atherosclerotic...

Merck and the Merck Manuals

Merck & Co., Inc., Kenilworth, NJ, USA is a global healthcare leader working to help the world be well. From developing new therapies that treat and prevent disease to helping people in need, we are committed to improving health and well-being around the world. The Merck Manual was first published in 1899 as a service to the community. The legacy of this great resource continues as the Merck Manual in the US and Canada and the MSD Manual outside of North America. Learn more about our commitment to Global Medical Knowledge.